Tetralogy of fallot with absent pulmonary valve syndrome; appropriate surgical strategies

Objective: To evaluate patients presenting with Tetralogy of Fallot with absent pulmonary valve syndrome to atertiary care hospital and their surgical management.Methods: The retrospective study was conducted at Congenital Cardiac Services, Aga Khan University Hospital,Karachi, Pakistan, and comprised data of Tetralogy of Fallot patients between April 2007 and June 2012. Data wasanalysed together with follow-up echocardiography. Variables assessed included demographics, imaging, operativetechnique, complications, post-operative recovery and follow-up echocardiography. SPSS 17 was used for statisticalanalysis.Results: Of the 204 patients, 6 (3%) had undergone surgical correction for Tetralogy of Fallot with absent pulmonaryvalve syndrome. All 6(100%) patients underwent complete repair. Median age for surgery was 8.5 years (range: 0.5-29 years). Of the different surgical strategies used, Contegra and Bioprosthetic valve placement had satisfactoryoutcome with minimal gradient at Right Ventricular Outflow Tract, good ventricular function and mild valvularregurgitation. One (16.6%) patient with Trans Annular Patch developed post-operative Right Ventricle OutflowTractgradient of 80mmHg withmoderate pulmonary regurgitation. One (16.6%) patient withmonocusp valve developedfree pulmonary regurgitation at 6 months. The other 4(66.6%) patients are currently free from any complications orre-intervention.Conclusion: Early surgery is preferred in symptomatic patients. The repair depends upon achieving integrity ofpulmonary circulation which is best achieved by using right ventricle to pulmonary artery conduit or inserting apulmonary valve.